tbuckley/cabot-search huggingface dataset:
P07 Not just simple hand eczema: a case of Jo-1-positive antisynthetase syndrome (‘mechanic’s hands’)
Authors: Ahmed Al-Tameemi, Sarah Elyoussfi, Raed El-Naes
Journal: British Journal of Dermatology (2023)

Abstract Antisynthetase syndrome is a rare inflammatory muscle disease related to dermatomyositis and polymyositis. Anti-Jo-1 antibodies are the most detected antibodies in antisynthetase syndrome. The main clinical features of antisynthetase syndrome are myositis, polyarthritis, interstitial lung disease, mechanic’s hands and Raynaud phenomenon. A 50-year-old man with a background of inflammatory muscle disease and interstitial lung disease, for which he is under the care of respiratory and rheumatology teams, was referred to the dermatology department by his general practitioner with refractory xerotic dry fissured skin on his fingers and feet. He reported itchy, sore and painful dry, cracked skin on both his hands, mainly on the fingertips. It did not affect the rest of his body. He trialled potent topical steroids and emollients, but his condition did not improve. He was on a reducing course of prednisolone 15 mg daily and on long-term oxygen therapy at home for interstitial lung disease. On clinical examination, he had an eczematous-looking eruption. The appearance of his hands was in keeping with ‘mechanic’s hands’. Together with the underlying diagnosis of interstitial lung disease, a diagnosis of antisynthetase syndrome was suspected. Blood tests revealed a fibrinogen of 5.39 g L−1 [reference interval (RI) 1.50–4.50], lupus creatine kinase of 1591 IU L−1 (RI 40–320), positive antibodies to Ro (SS-A) and positive antibodies to Jo-1. Jo-1 antibodies are found in dermatomyositis or polymyositis. Ro antibodies are associated with Sjogren syndrome (primary or secondary) or systemic lupus erythematosus, and serum may be antinuclear antibody negative. Therefore, we suspected a diagnosis of antisynthetase syndrome. A patch test showed a positive reaction to nickel, which was deemed to be in keeping with antisynthetase syndrome hand eruption, or ‘mechanic’s hands’. The patient was commenced on mycophenolate, a low dose of acitretin 10 mg and emollients and reported a significant improvement in symptoms. Repeat blood tests were scheduled for 3 months, including lipid profile and liver function test, for monitoring purposes. A follow-up was arranged for 12 weeks. He had a background of recurrent hospital attendance with pericardial effusions and chest symptoms and was tested for COVID-19 multiple times. This case demonstrates the importance of multidisciplinary coordination with rheumatology and respiratory colleagues in antisynthetase syndrome.
BT09 Clinical performance of an artificial intelligence-based medical device deployed within an urgent suspected skin cancer pathway
Authors: Rachel Jenkins, Christopher Felix Brewer, Dilraj Kalsi, Daniel Mullarkey
Journal: British Journal of Dermatology (2023)

Abstract A secondary care trust received over 2800 urgent suspected skin cancer referrals (USCRs) in 2021, 50% more than in 2016 (www.england.nhs.uk/statistics/statistical-work-areas/cancer-waiting-times), &amp;lt; 1 in 10 of which resulted in diagnosis of skin cancer (www.cancerdata.nhs.uk/cwt_conversion_and_detection). Judicious implementation of artificial intelligence (AI) may improve referral accuracy. We conducted a prospective, postdeployment, single-centre clinical performance review of a UK Conformity Assessed Class IIa-approved, AI-based, skin lesion analysis medical device intended for use in the screening, triage and assessment of skin lesions suspicious for skin cancer (AIaMD). The AIaMD is deployed at the trust as a decision-support tool following USCR from primary care, with a second read of all cases by a consultant dermatologist. Patients assessed by the two most recent versions of AIaMD from October 2021 to October 2022 were eligible for inclusion. Outcomes were confirmed from histology reports for cancerous lesions and histology reports or Consultant teledermatology assessment for noncancerous lesions. The AIaMD assessed 1363 lesions with outcomes confirmed, including 51 melanomas, 64 squamous cell carcinomas (SCCs), 96 basal cell carcinomas, 8 rare skin cancers and 244 premalignancies. The AIaMD referral sensitivity for melanoma, all skin cancer and premalignancy was 100% (n = 51/51), 99.5% (n = 218/219) and 96.3% (n = 235/244), respectively. The AIaMD benign lesion specificity was 55.9% (n = 434/777). The negative predictive value (how often AIaMD labelled lesions as premalignant/benign and correctly ruled out cancer) was 99.8% (n = 539/540). The conversion rate (the percentage of lesions classified as high risk by AIaMD that resulted in a diagnosis of melanoma or SCC) was 13.8% (n = 122/886), while the USCR conversion in the local clinical commissioning group was 9.9% in 2020–2021. The service discharged 9.4% (n = 239/2551) of patients during this time without using trust dermatologist capacity, with the potential to have discharged 18.3% (n = 467/2551) without a second read. This postmarket service evaluation reported the clinical outcomes of an AI device used for assessment of skin lesions. The pathway was sensitive, identifying 218 of 219 skin cancers, and specific, correctly identifying over half of benign lesions. Moreover, AIaMD was able to improve the conversion rate of USCRs. The integration of AIaMD into skin cancer diagnostic pathways could significantly improve the accuracy of USCRs. Conflicts of interest: these were commercial deployments of the AI, and three of the authors are employed by the AI provider.
BI23 Erythrodermic eczematous reactions to immunoglobulins
Authors: Adam Gold, Fazleenah Hussain, Paul Yesudian, Urvi Popli
Journal: British Journal of Dermatology (2023)

Abstract Intravenous immunoglobulins (IVIg) have been used in clinical practice for more than 50 years. They are used for dermatological indications such as dermatomyositis, pemphigus and Kawasaki disease. Although this is regarded as a safe treatment modality, it can produce adverse reactions in the skin. We report two patients with IVIg-induced skin changes. A 71-year-old man with no preceding skin disease was under neurology care for the treatment of chronic inflammatory demyelinating neuropathy (CIDP) with IVIg. He presented with an erythrodermic eczematous rash and was symptomatic with pain and pruritus. The rash started on the hands and had spread to the rest of the body 3 days after completion of his third course of IVIg. He was treated with topical clobetasol propionate. His skin settled over the course of a few weeks. The patient was keen to resume IVIg for CIDP; unfortunately, he developed a more severe and rapid reaction on retrial and IVIg had to be stopped. An 82-year-old man was given IVIg for CIDP. He denied any previous skin conditions or atopy. He received a 5-day course of IVIg followed by intravenous methylprednisolone 1 g once daily for 6 days. Four weeks later, he developed pompholyx on the palms and thereafter a widespread eczematous rash over the arms, abdomen, back and face. He was treated with topical clobetasol propionate to the trunk and limbs and clobetasone butyrate to the face. His rash and symptoms settled with topical treatment. Eczematous immunoglobulin reactions are often under-recognized. Reactions preferentially affect middle-aged men and those being treated for neurological disease. The clinical presentation is peculiar and distinctive, almost always involving the palmoplantar aspects with a dyshydrotic element. The reaction pattern starts within a few days to weeks of exposure to immunoglobulins and can become erythrodermic. Histology shows a spongiotic dermatitis and is not diagnostic. Immunoglobulin-associated eczema can persist even after the discontinuation of immunoglobulins. Recommended treatment depends on severity. If mild, continuing immunoglobulins with topical steroids should be considered. If the reaction persists or worsens, up to two switches of the immunoglobulin brand can be considered. In severe or persistent cases, methotrexate may be beneficial. The mechanism of an IVIg-induced skin reaction is not fully understood. The diagnosis of eczematous IVIg reaction is clinical, with a high index of suspicion required. The care of these patients requires collaboration between dermatologists and the physician prescribing the immunoglobulin, who may be unfamiliar with this adverse event.
BG11 Palmoplantar keratoderma with mutations in filaggrin and desmoglein-1 genes
Authors: Rebecca L McCarthy, Edel O’Toole
Journal: British Journal of Dermatology (2023)

Abstract Hereditary palmoplantar keratoderma (PPK) has many forms, and genetic testing can help distinguish diagnoses. This case demonstrates a presentation of PPK with an altered phenotype caused by a mutation in FLG. The patient was a 23-year-old woman referred with PPK since the age of 6 years. She reported dry skin on the back of her hands from infancy and no other dermatological conditions. She denied plantar pain except when there were fissures in the callus. There was no relevant past medical history, but a significant family history of individuals with PPK and curly hair, and sudden infant death (SID) in two siblings. Physical examination revealed focal plantar keratoderma bilaterally, curly hair, mild tongue leukokeratosis and generally dry skin on both legs. She had palmar hyperlinearity, small calluses on the palms and normal nails. She declined a skin biopsy. The patient was referred to cardiology to rule out cardiomyopathy associated with a desmoplakin variant. She had an unremarkable transthoracic echocardiography and cardiac magnetic resonance imaging. Twelve-lead electrocardiography showed sinus rhythm with biphasic T-wave in V2 and inverted V1. Clinically arrhythmogenic right ventricular cardiomyopathy (ARVC) was ruled out; however, given her significant family history of SID and PPK, panel testing for genes associated with ARVC was performed, which revealed a heterozygous variant of uncertain significance in MYBPC3 (p.Val639lle). This variant did not explain the patient’s PPK; therefore, a PPK panel was performed. Next-generation sequencing identified a heterozygous DSG1 [c430A&amp;gt;T p.(Arg.144*)] pathogenic variant and a heterozygous FLG [c2282_2285del p.(Ser761Cysfs*36)] pathogenic variant. The patient’s PPK was more severe on the plantar surface of the feet than one might expect with a DSG1 variant. This case demonstrates the interaction between two genetic variants known to be associated with skin conditions. Ichthyosis vulgaris is caused by FLG variants. Patients with heterozygous FLG variants are less severely affected and have reduced penetrance compared to homozygotes. Meanwhile, heterozygous pathogenic variants in DSG1 are associated with keratosis palmoplantaris striata I (MIM148700). We hypothesize that the coexistence of FLG and DSG1 variants may contribute to a more extensive keratoderma phenotype than usually seen with DSG1 variants. This case contributes to our understanding of PPK, demonstrates the value of genetic testing in dermatological conditions and describes a unique phenotype in the presence of multiple genetic variants. Further research is required to better understand the interaction between variants in PPK pathogenesis.
BT16 A service evaluation of dermatology Clinical Assessments, Services, Education and Support which aims to reduce referrals from primary to secondary care through peer review and education
Authors: Rishi Shah, Helen Story, Andrea Hepden, Helen Ramsay, Sarah Cockayne
Journal: British Journal of Dermatology (2023)

Abstract Clinical Assessments, Services, Education and Support (CASES) is a collaboration between primary and secondary care to aid management of routine referrals via the National Health Service e-Referral Service (eRS) by providing clinical support and education to primary care through peer review, advice and guidance from local general practitioners (GPs) since 2016. The service covers 10 specialities. The CASES is optional, and primary care can choose to bypass the service, referring directly to secondary care. Three GP peer reviewers work under the supervision of dermatology consultants. They review elective referrals from primary healthcare professionals (GPs, advanced clinical practitioners and physician associates) via the eRS. Referrals are either sent back to the referrer with advice and educational resources or sent on to secondary care, often with interim advice. Monthly mentoring meetings enable the assessors to discuss difficult cases. Data were recorded for dermatology referrals from January 2022 to December 2022. Within the 12-month period, 3940 referrals were processed via CASES (mean 328 referrals a month). Of the referrals received, 90.0% (n = 3545) were routinely referred on to secondary care and 1.4% (n = 55) required a 2-week wait. However, 6.9% (n = 273) were returned to the referrer with advice and guidance. Further information was requested for 1.1% (n = 44) of the referrals that had been returned to the referrer. Twenty-three (0.6%) had been referred to the wrong specialty. Lesions (n = 1835; 46.6%) made up the bulk of the referrals, alongside rashes referred for diagnosis (n = 573; 14.5%). Chronic disease management (acne, eczema, psoriasis, contact dermatitis, etc.) made up 36.1%. Overall, the results suggest that 6.9% (n = 273) of referrals were managed in primary care. Almost half of the referrals were related to lesions. We have not yet been able to establish the proportion of cases where referrals were returned with advice and guidance and were then later referred to secondary care for the same issue, which is currently a limitation. Secondary care dermatology is overwhelmed with the current workload and waiting times. Peer review services like CASES, using a digital platform like the eRS, have the potential to be extremely beneficial to help manage the workload. However, the sharing of information between primary and secondary care needs to be improved in our service, and we would aim to make images mandatory to enable triage, although our beleaguered primary care colleagues currently feel that this is not possible.
PA08 Do parents and carers of children with eczema understand topical steroid potency? Results of a patient survey
Authors: Celia Moss, Alice Lambert, Zahra Haider, Andrew Proctor
Journal: British Journal of Dermatology (2023)

Abstract Dermatologists consider topical corticosteroids (TCS) to be the first-line active treatment for most inflammatory skin disorders, including eczema. Well-known adverse effects can usually be avoided by careful prescribing. However, widespread public antipathy to TCS, sometimes amounting to steroid avoidance and phobia, can impair patient care. This fear is largely independent of steroid potency, even though there can be up to a 100-fold difference between the mildest and the most potent products. National Institute for Health and Care Excellence guidelines refer to the World Health Organization’s four-level grading system (mild, moderate, potent and very potent), but information about potency is never found on TCS packaging and rarely in patient information leaflets (Haider Z, Proctor A, Moss C. Potency of topical steroids should be clearly labelled on all packaging. Br J Dermatol 2023; 188:427–8). We surveyed parents and carers to investigate their knowledge and understanding of steroid potency. A SurveyMonkey® questionnaire was promoted by the National Eczema Society on social media, and by Eczema Outreach Support in a newsletter to supporters in January 2023. Of 984 responses received, 25% were from parents or carers of children with eczema. Of these, 97% knew that TCS could have different strengths (potencies), but 44% did not know how many different grades there were, with only 20% correctly answering four. Despite this, 77% of respondents felt ‘somewhat confident’ or ‘very confident’ that they knew how strong their cream was, but of those who were ‘very confident’, 29% did not know how many potency grades there were. Follow-up questions revealed that of those who had used these specific TCS, 93% knew that hydrocortisone is mild, but only 48% correctly identified Fucidin H as mild. Only 54% correctly identified DermovateTM as very potent. When asked ‘Where would you look to find out how strong your steroid creams and ointments are?’ most (69%) answered ‘on the packaging’. Ninety-seven per cent were in favour of clear potency labelling on all steroid creams and ointments. Sixty-two respondents provided free-text comments that were analysed thematically: 51% expressed confusion about TCS or a need for more information; 18% complained that general practitioners were not sufficiently knowledgeable; 20% voiced concerns about TCS, some vehemently, and most citing ‘topical steroid withdrawal’; 11% were positive about TCS and the information provided, two of whom used the ‘Aron regimen’. Several responses indicated confusion between percentage concentration (stated on the tube) and potency. These findings confirm the clinical impressions that parents of children with eczema have limited understanding of potency and need clearer information on the tube and packaging.
BI12 The incidence and survival of Kaposi sarcoma using national cancer registry data
Authors: Catarina Quaresma, Agnieszka Lapin, Sally Vernon, Agnieszka Tan, Zoe C Venables
Journal: British Journal of Dermatology (2023)

Abstract Kaposi sarcoma (KS) is a rare soft tissue endothelial neoplasm associated with human herpesvirus (HHV)-8 infection. Four types of KS are recognized, with the majority of cases in the West attributed to HIV-associated KS or, less frequently, to iatrogenic KS caused by systemic immunosuppression. Population-based data on KS epidemiology in England are lacking, with the last published evaluation dating back to the 1980s. More recently, data on KS epidemiology have been included in the National Cancer Registration and Analysis Service’s (NCRAS) ‘Get Data Out’ annual reports, with details published from 2013 to 2019. A nonsignificant reduction in the incidence rate of KS in England was observed from 2013 [0.271; 95% confidence interval (CI) 0.229–0.319] to 2019 (0.222, 95% CI 0.185–0.265; www.cancerdata.nhs.uk/getdataout/kaposi_sarcoma). This figure is slightly lower than the most recent reported crude incidence rates of KS in Europe from 2000 to 2007 (0.28) and significantly lower than that of the USA in 2017 (0.45) and globally in 2020 (0.439) (https://gco.iarc.fr/today/data/factsheets/cancers/19-Kaposi-sarcoma-fact-sheet.pdf). Data from NCRAS show that most cases of KS in England were diagnosed following a general practitioner referral (32.8%), outside the 2-week wait route (15.6%). A noteworthy proportion of cases (17.2%) were diagnosed following an emergency presentation to the emergency department, emergency clinician referrals or acute admissions. The 5-year net survival after the diagnosis of KS was stable at 81.3% (95% CI 73–89.5), 86.8% (95% CI 79.5–94.1) and 79.7% (95% CI 67.2–92.2) for 2013, 2014 and 2015, respectively (www.cancerdata.nhs.uk/getdataout/kaposi_sarcoma). These data provide a useful insight into the epidemiology of this rare cancer; however, it is probable that the true incidence of KS is under-reported, particularly in cases where there is no histological confirmation or reporting of KS on the death certificate, which may be more likely in patients who were HIV positive. Lack of knowledge of the underlying cause for KS is a major limitation. In conclusion, there is a paucity of published data on KS epidemiology in England. Further evaluation of KS subsets, patient demographics and comorbidity at diagnosis is needed to allow a better understanding of differences in the KS incidence in England in comparison with global figures.
DS21 Patients’ experience with one-stop skin cancer clinics
Authors: Mun Wai Lam, Richard Jerrom
Journal: British Journal of Dermatology (2023)

Abstract Dermatological surgery has been well integrated into the daily work of a dermatologist, especially in the management of skin cancers. Therefore, dermatologists have to be well prepared to consent patients for their respective surgical procedures. Consent is a medicolegal and ethical requirement prior to any procedure. The General Medical Council has produced guidance outlining seven principles of decision-making and consent. With increasing referrals of suspected skin cancers to secondary care dermatology, it is increasingly common for one-stop clinics to be introduced to tackle the sheer volume—clinics where patients are seen and treated on the same day. There are concerns that such models can diminish patient understanding and the validity of consent. Mr Justice Kearns of the Supreme Court of Ireland stated: ‘As a general principle of good practice, if the treatment is not urgent, patients should be given plenty of time to think about their options before they consent to treatment and be encouraged to ask further questions’. Our department has recently introduced a one-stop skin cancer clinic, and we have conducted a survey to compare patient experiences in those who had same-day skin surgery and those who had a prebooked surgical appointment. The data were collected over a period of 3 months with a total of 80 respondents. All respondents expressed that they had complete understanding of their procedures, 23 of whom had same-day surgery. All 23 respondents who had same-day surgery were comfortable to have same-day surgery, with only six expressing that they felt anxious. When asked if the respondents preferred to have same-day surgery or surgery on other days, 21 of 23 respondents who had same-day surgery preferred to have it on the same day, whereas 34 of 57 of the respondents who had different-day surgery would rather have their surgery on the same day. A total of 69% preferred to have same-day surgery, with three expressing no preference. Our findings reveal that the majority of the respondents preferred to have their procedures on the same day as their clinic consultation and all expressed an adequate understanding of their procedure and felt that they have been given sufficient time to understand and weigh up their options. This suggests that patients have adequate understanding of their procedures, despite being consented and treated on the same day, while also improving their patient experience in a busy department.